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Objective: This study was designed to compare the clinical manifestations, laboratory tests, etiology, and prognosis of children with acute rhabdomyolysis (RM) at various ages. This study was designed to analyze the risk factors for acute kidney injury (AKI) in children with RM and to identify the role of neuromuscular and autoimmune disease in children with RM. Methods: Clinical data for 55 children with RM were collected and statistically analyzed. Patients were stratified to an infant group (G1) (age <1 year), preschool group (G2) (age 1-6 year), school-age group (G3) (age 7-11 year), and an adolescent group (G4) (age 12-16 year). Results: The top three clinical manifestations were dark urine (52.7%), myalgia (38.2%), and fever (23.8%). Patients in G1 had fever (71.4%), vomiting (77.8%), and urinalysis abnormalities (14.3%), without triad clinical manifestations. Fifty percent of patients in G4 group had myalgia; 70.8% had dark urine; 75% had abnormal urine tests. The most common cause in each age group was as follows: sepsis (57.1%) in G1; hereditary neuromuscular diseases (44.4%) in G2; immune diseases (40%) in G3; strenuous exercise (50%) in G4. Logistic regression analysis shown that AKI was not corelated with age, gender, or peak creatine phosphokinase. AKI was, however, associated with presence of an electrolyte disorder. Conclusion: The clinical manifestations and laboratory findings in infants with acute RM are not typical and need to be taken seriously. The presence of an electrolyte disorder is a risk factor for AKI in children with RM. The most common pathogenesis of RM varies among age groups. Congenital hereditary metabolic disease and immune diseases should not be ignored as a cause of RM in children.

Mutations in TAB2 (transforming growth factor β activated kinase 1 binding protein 2) have been implicated in the pathogenesis of dilated cardiomyopathy and/or congenital heart disease in humans, but the underlying mechanisms are currently unknown. Here we identified an indispensable role for TAB2 in regulating myocardial homeostasis and remodeling by suppressing RIPK1 (receptor-interacting protein kinase 1) activation and RIPK1-dependent apoptosis and necroptosis. Cardiomyocyte-specific deletion of Tab2 in mice triggered dilated cardiomyopathy with massive apoptotic and necroptotic cell death. Moreover, Tab2-deficient mice were also predisposed to myocardial injury and adverse remodeling following pathological stress. In cardiomyocytes, deletion of TAB2, but not its close homologue TAB3, promoted TNFa-induced apoptosis and necroptosis, which was rescued by forced activation of TAK1 or inhibition of RIPK1 kinase activity. Mechanistically, TAB2 critically mediates RIPK1 phosphorylation at Ser321 via a TAK1-dependent mechanism, which prevents RIPK1 kinase activation and the formation of RIPK1-FADD-caspase-8 apoptotic complex or RIPK1-RIPK3 necroptotic complex. Strikingly, genetic inactivation of RIPK1 with Ripk1-K45A knock-in effectively rescued cardiac remodeling and dysfunction in Tab2-deficient mice. Together, these data demonstrate that TAB2 is a key regulator of myocardial homeostasis and remodeling by suppressing RIPK1-dependent apoptosis and necroptosis. Our results also suggest that targeting RIPK1-mediated cell death signaling may represent a promising therapeutic strategy for TAB2 deficiency-induced dilated cardiomyopathy.

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The aim of this study was to explore the impact of 3% hypertonic saline (HS) intragastric administration for patients who underwent upper gastrointestinal surgery.During the postoperative period, 3% HS has been suggested as a means to improve the intestinal edema and reduce gastrointestinal complications.The medical records of 111 patients with HS intragastric administration following upper gastrointestinal surgery and 268 patients, served as control, were reviewed retrospectively. Propensity score matching was performed to adjust for selected baseline variables. Clinical outcomes, including early gastrointestinal function recovery, postoperative complications, and length of hospital stay, were compared according to the HS intragastric administration or not.HS intragastric administration was associated with prompt postoperative gastrointestinal function recovery, including first flatus (risk ratio [RR], 1.32; 95% confidence interval [CI], 0.89–1.65; P = 0.048) and feeding within 3 postoperative days (RR (95% CI), 0.57 (0.49–0.77); P = 0.036). Early ileus occurred in 25 of 108 patients with HS treatment versus 36 of 108 patients without HS treatment (RR (95% CI), 1.43 (0.63–2.15); P = 0.065). The patients with HS experienced a lower overall postoperative complication (odds ratio [OD] 0.57; 95% CI, 0.33–1.09; P = 0.063), including trend toward a decrease for infectious complications (15[13.9] vs 23[21.3]; P = 0.11; OD, 0.59; 95% CI, 0.29–1.22). There was a decreased incidence of anastomotic leakage (1[0.9] vs 7[6.5]; P = 0.033) and postoperative ileuas (5[4.6%] vs 11[10.2%]; P = 0.096) in the HS administration patients.Our study demonstrated beneficial postoperative clinical effects of HS intragastric administration in patients who had undergone upper gastrointestinal surgery, such as prompt postoperative gastrointestinal function recovery and reduced overall postoperative complications, which may be attributed to a reduced intestinal edema.

Background: To investigate the efficacy of the ketogenic diet (KD) and anti-epileptic drugs (AEDs) on children with refractory epilepsy (RE), and to analyze the effects of gender, therapeutic time, age, type of epilepsy syndrome, number of epileptic seizure types, and number of AEDs on the efficacy of the KD. A prospective, non-randomized, controlled cohort study was used and a total of 200 children with RE who were hospitalized in the Department of Pediatric Neurology, Children's Hospital of Chongqing Medical of the KD, and the results showed no statistical significance (P>0.05).Conclusions: KD therapy reduces the frequency of seizures in children with RE compared with AEDs alone. In the KD group, the gender, therapeutic time, age, type of epilepsy syndrome, number of epileptic seizure types, and number of anti-epileptic drugs had no significant effect on the efficacy of the KD diet.

Rationale:Autoimmune encephalitis related to many antibodies against neuronal cell surface or synaptic proteins, it is increasingly recognized as the cause of a variety of neuropsychiatric syndromes.Patient concerns:The two pediatric cases were about autoimmune encephalitis with rare complication. One patient was a 11-year-old girl and was diagnosed with Voltage-Gated Potassium Channel complex (VGKC) antibody-mediated encephalitis with rhabdomyolysis; the other was also a 11-year-old girl and was diagnosed with anti- N-methyl-D-aspartate receptor (NMDAR) encephalitis.Diagnoses:Both patients were diagnosed as autoimmune encephalitis with rare complication.Interventions:Intravenous methylprednisolone, oral prednisone and intravenous immunoglobulin was administered to both patients.Outcomes:One patient was discharged after a half month's hospitalization; the other was finally with intestinal function failure, gradually developed multiple organ failure, and eventually died.Lessons:The pathogenic mechanism of autoimmune encephalitis associated with autoimmune disease is not fully understood, but may be related to a common immune pathological mechanism with variance in susceptibility caused by genetic or environmental factors.